Sleep-disordered breathing and effects of non-invasive ventilation on objective sleep and nocturnal respiration in patients with myotonic dystrophy type I
Sleep-disordered breathing and effects of non-invasive ventilation on objective sleep and nocturnal respiration in patients with myotonic dystrophy type I
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DOI：
10.1016/j.nmd.2019.02.006
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发表时间：
2019-04-01
影响因子：
2.8
通讯作者：
Boentert, Matthias
中科院分区：
文献类型：
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作者：
Spiesshoefer, Jens;Runte, Maya;Boentert, Matthias
I 型强直性肌营养不良 (DM1) 患者可能会出现夜间通气不足，需要无创通气。关于长期坚持无创通气或睡眠和通气结果的数据很少。我们回顾性收集了 36 名患有睡眠相关症状的成年患者（42.9 +/- 12.5 岁，20 名女性）的基线多导睡眠图和二氧化碳测定结果，以及接受无创通气治疗的患者的后续睡眠研究记录。 33 名患者 (91.7%) 发现睡眠呼吸障碍，其中 8 名患者 (22.2%) 患有白天高碳酸血症。 26 名患者（72.2%）经皮二氧化碳测定显示夜间通气不足。血氧饱和度检测夜间通气不足的灵敏度仅为 0.38。 28 名患者 (77.8%) 出现睡眠呼吸暂停，主要为阻塞性 (n = 8)、中枢性 (n = 9) 或“混合性”(n = 11)。 32 名患者开始接受无创通气，在治疗的第一个晚上显着改善了通气和氧合。随访显示，在长达 52 个月的时间内，血氧饱和度和碳酸血症稳定，睡眠结果没有恶化。治疗依从性为低至中度，个体间差异很大。睡眠呼吸障碍在主诉白天嗜睡的成年 DM1 患者中非常普遍，无创通气可显着、快速且持续地改善夜间气体交换。在检测夜间通气不足方面，二氧化碳测定法优于血氧测定法。坚持无创通气仍然是 DM1 的一个主要问题。长期治疗效益应单独评估。 (C) 2019 Elsevier B.V. 保留所有权利。
Patients with myotonic dystrophy type I (DM1) may develop nocturnal hypoventilation, requiring non-invasive ventilation. Data on long-term adherence to non-invasive ventilation, or sleep and ventilation outcomes are scarce. We retrospectively collected baseline polysomnography and capnometry results from 36 adult patients with sleep-related symptoms (42.9 +/- 12.5 years, 20 female), plus follow-up sleep study records from those treated with non-invasive ventilation. Sleep-disordered breathing was found in 33 patients (91.7%) including 8 (22.2%) with daytime hypercapnia. Twenty-six patients (72.2%) showed nocturnal hypoventilation on transcutaneous capnometry. The sensitivity of oximetry to detect nocturnal hypoventilation was only 0.38. Twenty-eight patients (77.8%) showed sleep apnea, which was predominantly obstructive (n = 8), central (n = 9), or "mixed" (n = 11). Thirty-two patients were initiated on non-invasive ventilation which significantly improved ventilation and oxygenation in the first night of treatment. Follow-up revealed stable normoxia and normocapnia without deterioration of sleep outcomes for up to 52 months. Adherence to treatment was low to moderate, with substantial inter-individual variability.Sleep disordered breathing is highly prevalent in adult DM1 patients complaining of daytime sleepiness, and non-invasive ventilation significantly, rapidly and persistently improves nocturnal gas exchange. Capnometry is superior to oximetry for detection of nocturnal hypoventilation. Adherence to non-invasive ventilation remains a major issue in DM1. and long-term treatment benefits should be individually assessed. (C) 2019 Elsevier B.V. All rights reserved.